Heart 1999;82:30-33 ( July )

Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects

M Vogel^a, F Berger^a, A Kramer^a, V Alexi-Meshkishvili^b, P E Lange^a

^a Department of Congenital Heart Disease, Deutsches Herzzentrum, Augustenburger Platz 1, D-13353 Berlin, Germany, ^b Department of Surgery, Deutsches Herzzentrum

Correspondence to: Prof Dr Vogel.

Accepted for publication 16 February 1999

OBJECTIVETo examine the incidence of raised pulmonary artery pressure and resistance in adults with isolated atrial septal defect within the oval fossa (so called secundum defect) or sinus venosus defect.
DESIGNA historical, retrospective, unrandomised study.
SETTINGA tertiary referral centre.
METHODSCardiac catheterisation was performed in all patients, with measurement of pulmonary artery pressure and resistance. Pulmonary to systemic flow ratio was calculated using the Fick principle. Pulmonary hypertension was defined as mean pulmonary artery pressure > 30 mm Hg, and increased resistance as an Rp/Rs ratio > 0.3.
PATIENTSAll patients with a secundum atrial septal or sinus venosus defect who presented between July 1988 and December 1997 were enrolled in the study.
RESULTSPulmonary artery pressure and resistance in the patients with sinus venosus defect (n = 31) was higher than in patients with atrial septal defect (n = 138). Pulmonary hypertension was present in 26% of patients with sinus venosus and in 9% of patients with atrial septal defect. The incidence of raised pulmonary vascular resistance was 16% in patients with sinus venosus and 4% in patients with atrial septal defect. The increase in resistance occurred at a younger age in sinus venosus defect than in atrial septal defect.
CONCLUSIONSPatients with sinus venosus defect have higher pulmonary pressures and resistances and develop these complications at younger age than patients with atrial septal defects. Thus they should be managed differently than patients with "simple" atrial septal defects.


Keywords: sinus venosus defect; atrial septal defect; pulmonary hypertension; congenital heart defects

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© 1999 by Heart
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